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Brugada syndrome is thought to account for about one fourth of suddencardiacdeaths in individuals with structurally normal heart. It is seldom done in pediatric age group. Yet, SCN5A variants are identified in only about one fifth of persons with Brugada syndrome. With proper precautions, risk can be reduced.
This discussion comes from this previous post: Hyperthermia and ST Elevation Discussion Brugada Type 1 ECG changes are associated with suddencardiacdeath (SCD) and the occurrence of ventricular dysrhythmias. Pediatric and elderly patients were more predisposed to developing an arrhythmic event in the setting of fever [7].
The limb lead abnormalities appear to be part of the Brugada pattern, as described in this article: Inferior and Lateral Electrocardiographic RepolarizationAbnormalities in Brugada Syndrome Discussion Brugada Type 1 ECG changes are associated with suddencardiacdeath (SCD) and the occurrence of ventricular dysrhythmias.
However, according to these diagnostic criteria (JACC 2011; 57(7):802), it is a Bazett corrected QT of less than 330-370, depending on other diagnostic criteria, including 1) h/o cardiacarrest, 2) sudden syncope, 3) family hx of sudden unexplained arrest at age less than 40, 4) family hx of SQTS.
BACKGROUND:Sudden cardiacdeath is the most common cause of death in childhood hypertrophic cardiomyopathy (HCM). Recently, 2 risk scores have been developed to estimate the 5-year risk of suddencardiacdeath. HCM Risk-Kids and PRIMaCY risk scores were calculated at diagnosis and during follow-up.
Further history later: This patient personally has no further high risk features (syncope / presyncope), but her mother had suddencardiacarrest in sleep. Twenty-one percent (18 of 88) had a family history of suddencardiacdeath and 26.4% (14 of 53) carried a pathogenic SCN5A mutation.
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